Overall ovjective of the proposed research is to investigate the pathogenetic mechanism of the lesions in metabolic neurological disorders in infancy and childhood using genetically determined or experimentally induced animal models. The animal models to be investigated for abnormal myelination are neurological mutant mice with myelin deficiency, quaking, experimentally induced copper deficient rats with hypomyelination and demyelination induced by ethidium bromide in rats. For the investigation on neuronal abnormalities, cerebellum of quaking mice and experimentally induced neuronal storage by chlorochine, chlorphentermine and AY9944 will be used. The study will be basically focused on the morphological alteration during the development of neuropathological lesions using light and electron microscopies, Golgi preparation, tissue culture and freeze-fracture technique.